HighQC™ Human IPSC From Fibroblast-Phenylketonuria

Description

HighQC™ Human IPSC From Fibroblast-Phenylketonuria (PKU) is a disease-specific human induced pluripotent stem cell line derived from dermal fibroblasts of a donor diagnosed with Phenylketonuria (PKU), an autosomal recessive inborn error of metabolism caused by mutations in the PAH gene leading to deficient phenylalanine hydroxylase activity. The resulting iPSCs (induced pluripotent stem cells) exhibit adherent growth with a high nucleus-to-cytoplasm ratio. They retain pluripotency and model PKU-related metabolic traits. These cells retain the fundamental capacity for trilineage differentiation into cell types of the ectoderm, mesoderm, and endoderm germ layers. They express the core pluripotency marker SSEA-4. The cells undergo rigorous screening and isolation procedures, and are rigorously tested to ensure they are free of contamination from HIV-1, HBV, HCV, Syphilis, Mycoplasma, Fungi, Yeast, and Bacteria.